Congenital atresia of unilateral pulmonary veins associated with a single ventricle - A rare case report and literature review

Hsing Yuan Lee, Betau Hwang*, Pi Chang Lee, Sheng Ling Jan, C. C.Laura Meng

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

8 Scopus citations

Abstract

Congenital atresia or extreme hypoplasia of individual pulmonary veins is a rare condition that is usually asymptomatic if it only involves 1 or 2 segments of the lungs. It may be fatal if it occurs in combination with other complex cardiac defects. The patients often present with recurrent pulmonary infections, hemoptysis, or cyanosis in the latter case. A definitive diagnosis can be made by cardiac catheterization with selective pulmonary wedge angiography. The treatment of this condition is a challenge and is controversial, and the prognosis is usually poor. We describe the case of a male infant who suffered from shortness of breath and cyanosis since birth. On echocardiography and cardiac catheterization, he was found to have a complex congenital heart disease with a single ventricle (right ventricle morphology), mitral atresia, large atrial septal defect, and atresia of the right pulmonary veins; severe pulmonary hypertension was also observed. He underwent pulmonary artery banding at the age of 2.5 months, but he died because of a pulmonary infection at the age of 6 months. In this report, we present this rare case and review the previous literature.

Original languageEnglish
Pages (from-to)1544-1546
Number of pages3
JournalCirculation Journal
Volume72
Issue number9
DOIs
StatePublished - 2008

Keywords

  • Congenital heart disease
  • Pulmonary vein atresia
  • Single ventricle

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